Aggressive NK-cell leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [4] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [5]

Synonyms and keywords: ANKL

Overview

Aggressive NK-cell leukemia is a disease with an aggressive, systemic proliferation of natural killer cells (NK cells) and a rapidly declining clinical course. There is no classification system for aggressive NK-cell leukemia. The pathogenesis of aggressive NK-cell leukemia is characterized by the proliferation of natural killer cells. Aggressive NK-cell leukemia is very uncommon, and is most commonly seen among middle aged adults. The most common risk factors in the development of aggressive NK-cell leukemia is the Epstein-Barr virus (EBV) infection. The majority of patients with aggressive NK-cell leukemia are symptomatic at diagnosis. Early clinical features include fatigue, night sweats, and fever. Physical examination among patients with aggressive NK-cell leukemia may be remarkable for hepatosplenomegaly. Aggressive NK-cell leukemia may also be diagnosed using bone marrow biopsy, findings include: extensive marrow replacement by leukemic cells and reactive histiocytes displaying hemophagocytosis. The mainstay of therapy for aggressive NK-cell leukemia is anthracycline-containing chemotherapy.

Classification

• There is no classification system for aggressive NK-cell leukemia.

Pathophysiology

• The pathogenesis of aggressive NK-cell leukemia is characterized by the aggressive, systemic proliferation of natural killer cells.
• The mutation in oncogene p53 has been associated with the development of aggressive NK-cell leukemia.
• On gross pathology, characteristic findings of aggressive NK-cell leukemia, include:

• No remarkable findings

• On microscopic histopathological analysis, characteristic findings of aggressive NK-cell leukemia, include:

• Large cells with abundant blue cytoplasm
• Azurophilic granules
• Irregular nuclei
• Open chromatin
• Distinct nucleoli
• CD11b and CD16 show variable expression

• The table below demonstrates the immunophenotype for patients with aggressive NK-cell leukemia.

Status	Antigens
Positive	CD2, CD3ε, CD56, perforin, granzyme B, TIA-1, CCR5
Negative	CD57

Causes

• There are no established causes for aggressive NK-cell leukemia.

Differentiating Aggressive NK-cell Leukemia from Other Diseases

• Aggressive NK-cell leukemia must be differentiated from other diseases that cause fever, fatigue, and lymphadenopathy such as:

• Human immunodeficiency virus
• Chronic neutrophilic leukemia
• Juvenile myelomonocytic leukemia

Epidemiology and Demographics

• Aggressive NK-cell leukemia is very uncommon.

Age

• Aggressive NK-cell leukemia is more commonly observed among middle aged adults.

Gender

• Aggressive NK-cell leukemia affects men and women equally.

Race

• Aggressive NK-cell leukemia usually affects individuals of the Asians race.

Risk Factors

• Common risk factors in the development of aggressive NK-cell leukemia, include:

• Epstein-Barr virus (EBV) infection

Natural History, Complications and Prognosis

• The majority of patients with aggressive NK-cell leukemia are symptomatic at diagnosis.
• Early clinical features include fatigue, night sweats, and fever.
• If left untreated, patients with aggressive NK-cell leukemia may progress to develop recurrent infections.
• Common complications of aggressive NK-cell leukemia, include:

• Coagulopathies
• Hemophagocytic syndrome
• Multiple organ failure
• Myelofibrosis

• Prognosis is generally poor, and the median survival rate of patients with aggressive NK-cell leukemia is approximately 12 months.

Diagnosis

Symptoms

• Aggressive NK-cell leukemia is usually asymptomatic.
• Symptoms of aggressive NK-cell leukemia may include the following:

• Fever
• Swelling in the lymph nodes in the neck
• Night sweats
• Persistent fatigue
• Loss of appetite
• Nausea
• Vomiting

Physical Examination

• Patients with aggressive NK-cell leukemia usually appear pale and malnourished.
• Physical examination may be remarkable for:

• Hepatoesplenomegaly

Laboratory Findings

• Laboratory findings consistent with the diagnosis of aggressive NK-cell leukemia, include:

Peripheral Blood Smear

• Large granular lymphocyte (LGL)
• Azurophilic granules and nucleoli of varying prominence
• Nuclei may be irregular and hyperchromatic

Imaging Findings

• There are no imaging findings associated with aggressive NK-cell leukemia.

Other Diagnostic Studies

• Aggressive NK-cell leukemia may also be diagnosed using bone marrow biopsy.
• Findings on bone marrow biopsy, include:

• Extensive marrow replacement by leukemic cells
• Reactive histiocytes displaying hemophagocytosis

Treatment

Medical Therapy

• The mainstay of therapy for aggressive NK-cell leukemia is anthracycline-containing chemotherapy.
• Other novel treatments may include pralatrexate.

Surgery

• Surgery is not recommended among patients with aggressive NK-cell leukemia.

Prevention

• There are no primary preventive measures available for aggressive NK-cell leukemia.

Historical Perspective

• [Disease name] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
• In [year], [gene] mutations were first identified in the pathogenesis of [disease name].
• In [year], the first [discovery] was developed by [scientist] to treat/diagnose [disease name].

Classification

• [Disease name] may be classified according to [classification method] into [number] subtypes/groups:

• [group1]
• [group2]
• [group3]

• Other variants of [disease name] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• The pathogenesis of [disease name] is characterized by [feature1], [feature2], and [feature3].
• The [gene name] gene/Mutation in [gene name] has been associated with the development of [disease name], involving the [molecular pathway] pathway.
• On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
• On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Differentiating [disease name] from other Diseases

• [Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of [disease name] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [disease name] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop [disease name].

• [Disease name] is more commonly observed among patients aged [age range] years old.
• [Disease name] is more commonly observed among [elderly patients/young patients/children].

Gender

• [Disease name] affects men and women equally.

• [Gender 1] are more commonly affected with [disease name] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [disease name].

• [Disease name] usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [disease name].

Risk Factors

• Common risk factors in the development of [disease name] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

• The majority of patients with [disease name] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [disease name] is approximately [#%].

Diagnosis

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:

• [criterion 1]
• [criterion 2]
• [criterion 3]
• [criterion 4]

Symptoms

• [Disease name] is usually asymptomatic.
• Symptoms of [disease name] may include the following:

• [symptom 1]
• [symptom 2]
• [symptom 3]
• [symptom 4]
• [symptom 5]
• [symptom 6]

Physical Examination

• Patients with [disease name] usually appear [general appearance].
• Physical examination may be remarkable for:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

Laboratory Findings

• There are no specific laboratory findings associated with [disease name].

• A [positive/negative] [test name] is diagnostic of [disease name].
• An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of [disease name].
• Other laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

• There are no [imaging study] findings associated with [disease name].

• [Imaging study 1] is the imaging modality of choice for [disease name].
• On [imaging study 1], [disease name] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• [Disease name] may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for [disease name]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [disease name] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action 1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [disease name].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [disease name].
• [Surgical procedure] can only be performed for patients with [disease stage] [disease name].

Prevention

• There are no primary preventive measures available for [disease name].

• Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [disease name] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].